Immune-Mediated Skin Disorders | MyMerci
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Immune-Mediated Skin Disorders

Unit 15 · Topic 85Immune-Mediated Skin Disorders
1.Overview & Pathophysiology

These disorders result from an overactive or misdirected immune response in the skin. Some are limited to the skin; others are skin signs of systemic autoimmune disease; a few are life-threatening drug reactions.

DisorderMechanismHallmark
PsoriasisT-cell–driven (IL-17/IL-23) inflammation → keratinocytes turn over in days instead of weeksWell-demarcated red plaques with silvery scale on elbows, knees, scalp, lower back; nail pitting; Koebner phenomenon (lesions at sites of injury); psoriatic arthritis in some
Atopic dermatitis (eczema)Skin barrier defect (e.g., filaggrin) + type 2 (IL-4/IL-13) inflammationIntensely itchy, dry, red patches; flexural areas in older children and adults; lichenification; linked to asthma and allergic rhinitis
Contact dermatitisIrritant (direct damage, e.g., detergents, frequent handwashing) or allergic (delayed type IV hypersensitivity, e.g., nickel, fragrance, latex accelerators, poison ivy)Rash limited to contact area; vesicles in allergic type
UrticariaMast cell release of histamine → leaky dermal vesselsRaised, itchy wheals that move and fade within 24 hours; acute (under 6 weeks) or chronic
AngioedemaDeeper swelling; histamine-mediated (with urticaria) or bradykinin-mediated (ACE inhibitors, hereditary)Swelling of lips, tongue, eyelids, airway
SJS / TENSevere cytotoxic T-cell reaction, usually to a drug, causing epidermal death and detachmentFever and flu-like prodrome → painful dusky rash, blisters, mucosal erosions (mouth, eyes, genitals), positive Nikolsky sign

Autoimmune blistering diseases (contrast with SJS/TEN)

DiseaseFeatures
Pemphigus vulgarisAntibodies against connections between epidermal cells → flaccid blisters and painful oral erosions; Nikolsky sign positive; treated with systemic corticosteroids and rituximab; fluid loss and infection risk
Bullous pemphigoidAntibodies at the basement membrane → tense blisters on itchy skin, mostly in older adults; Nikolsky negative; mucosa rarely involved; potent topical or systemic corticosteroids

SJS/TEN spectrum by body surface area (BSA) detached: SJS under 10%, overlap 10–30%, TEN over 30%.

Common culprit drugs: allopurinol, carbamazepine, lamotrigine, phenytoin, sulfonamides (including TMP-SMX), nevirapine, oxicam NSAIDs. Onset is usually 4–28 days after starting the drug. Genetic risk: HLA-B*15:02 with carbamazepine (common in many Southeast Asian populations) and HLA-B*58:01 with allopurinol.

Skin signs of systemic autoimmune disease

DiseaseSkin findings
Systemic lupus erythematosus (SLE)Malar ("butterfly") rash over cheeks and nose sparing the nasolabial folds, photosensitivity, discoid lesions, oral ulcers, hair loss
Systemic sclerosis (scleroderma)Excess collagen → thick, tight, shiny skin, mask-like face with reduced expression, small mouth, Raynaud phenomenon, digital ulcers
DermatomyositisHeliotrope rash (violet eyelids) and Gottron papules over knuckles, plus proximal muscle weakness; associated with cancer in adults
Behçet diseaseRecurrent oral and genital ulcers, skin lesions (erythema nodosum, acne-like lesions), uveitis, pathergy

Raynaud phenomenon: cold or stress triggers digital vasospasm → white (pallor) → blue (cyanosis) → red (reactive hyperemia).

2.Assessment Findings
  • Distribution, morphology, scale, vesicles, itching severity, sleep disruption
  • Mucosal involvement and skin that peels with light pressure (SJS/TEN) — emergency
  • Airway: lip, tongue, or throat swelling, hoarseness, stridor (angioedema)
  • Medication history (new drugs in the last 8 weeks), exposures, allergies
  • Joint pain, fatigue, muscle weakness, dysphagia (systemic disease)
  • Psychosocial impact: visible lesions affect self-image, work, and relationships
3.Diagnostics
TestUse
Clinical diagnosisPsoriasis, eczema, urticaria
Patch testingIdentifies allergens in allergic contact dermatitis
Skin biopsyConfirms SJS/TEN, lupus, dermatomyositis
SCORTENPredicts mortality in SJS/TEN
ANA, anti-dsDNA, complementSLE
Anti-Scl-70, anti-centromereScleroderma
CK, aldolase, myositis antibodiesDermatomyositis; age-appropriate cancer screening
TB screening, hepatitis serologyBefore biologic, JAK inhibitor, or methotrexate therapy
4.Medical Management

Psoriasis

  • Mild: topical corticosteroids, vitamin D analogs (calcipotriene), tar, keratolytics; scalp: steroid solutions, foams, or shampoos
  • Moderate–severe: phototherapy (narrowband UVB), methotrexate, cyclosporine, acitretin, apremilast, deucravacitinib, biologics (TNF, IL-17, IL-23 inhibitors)

Atopic dermatitis

  • Daily emollients and gentle skin care are the foundation
  • Topical corticosteroids for flares; topical calcineurin inhibitors (tacrolimus, pimecrolimus) for face and folds; topical PDE-4 or JAK inhibitors
  • Moderate–severe: dupilumab, tralokinumab, lebrikizumab, nemolizumab, oral JAK inhibitors, phototherapy

Contact dermatitis: identify and avoid the trigger; topical steroids; oral steroid taper for extensive allergic reactions.

Urticaria / angioedema

  • Second-generation H1 antihistamines (cetirizine, loratadine, fexofenadine) first-line; dose can be increased up to four-fold for chronic urticaria; omalizumab if refractory
  • Anaphylaxis or airway swelling: IM epinephrine first
  • ACE-inhibitor angioedema: stop the ACE inhibitor; antihistamines and steroids are often ineffective (bradykinin-mediated)

SJS/TEN

  • Stop the suspected drug immediately
  • Transfer to a burn unit or ICU; supportive care similar to burn care: fluids, wound care, temperature control, nutrition, pain control
  • Daily ophthalmology review; mucosal care; immunomodulators (cyclosporine, etanercept, IVIG) are used in some centers

Systemic autoimmune disease: hydroxychloroquine and sun protection (lupus); calcium channel blockers for Raynaud; immunosuppressants per specialist.

Drug safety

DrugKey safety points
Topical corticosteroidsSkin atrophy, striae, telangiectasia; avoid potent agents on face, groin, and axillae; use fingertip-unit amounts; long use over large areas can suppress the adrenal axis
Topical calcineurin inhibitorsBurning at start; boxed warning (theoretical malignancy); avoid under age 2
MethotrexateOnce-weekly dosing — daily dosing errors are fatal; folic acid; monitor CBC, liver, kidney; teratogenic; avoid alcohol; interactions with NSAIDs and TMP-SMX
BiologicsSerious infections; screen for TB and hepatitis B first; no live vaccines; injection-site reactions; dupilumab causes conjunctivitis
Oral JAK inhibitorsBoxed warnings: serious infection, cardiovascular events, thrombosis, malignancy, death
CyclosporineNephrotoxicity, hypertension; many interactions
AcitretinTeratogenic — avoid pregnancy for 3 years after stopping; people who can become pregnant must avoid alcohol during and for 2 months after therapy (forms long-acting etretinate); no blood donation during and for 3 years after; monitor lipids and liver tests
IsotretinoinTeratogenic; US iPLEDGE program; avoid pregnancy and blood donation for 1 month after (see Topic 88)
First-generation antihistamines (diphenhydramine, hydroxyzine)Sedation — no driving or operating machinery; avoid alcohol; anticholinergic effects; avoid in older adults
Second-generation antihistaminesLess sedating; adjust for kidney impairment
HydroxychloroquineRetinal toxicity — baseline and yearly eye exams after 5 years; QT prolongation
Epinephrine IM0.01 mg/kg of 1 mg/mL, maximum 0.5 mg adult, anterolateral thigh; repeat every 5–15 min as needed
5.Nursing Interventions

Listed in priority order.

  1. Airway — angioedema or anaphylaxis: call for help, give IM epinephrine, oxygen, prepare for airway management
  2. SJS/TEN — treat like a major burn
    • Stop the drug, notify the provider
    • Fluid and electrolyte replacement, monitor urine output
    • Protect denuded skin: non-adherent dressings, no adhesive tape, handle gently with draw sheets
    • Warm room, strict asepsis, monitor for sepsis
    • Eye care (lubricants, ophthalmology), mouth care, pain control, nutrition
  3. Itch control and skin protection
    • Keep nails short; discourage scratching and rubbing
    • Cool compresses, lukewarm baths, fragrance-free emollients applied right after bathing
    • Loose cotton clothing
  4. Medication administration and monitoring (see table)
  5. Psychosocial support — body image, anxiety, sleep; peer support groups
6.Client Education
  • Bathe in lukewarm (not hot) water with mild, fragrance-free cleansers; pat dry; moisturize within minutes
  • Do not rub or scratch; keep nails short; wear cotton gloves at night if needed
  • Identify and avoid triggers: allergens, irritants, heat, stress, certain drugs
  • SLE: avoid sun exposure, use broad-spectrum sunscreen and protective clothing; wash rash areas gently with lukewarm water — avoid hot water and frequent scrubbing
  • Raynaud: keep the whole body warm, wear gloves, stop smoking, avoid caffeine and vasoconstricting decongestants, manage stress
  • Psoriasis: apply scalp products as directed (part hair, apply to lesions); sunlight in small amounts may help but avoid burns
  • Antihistamines: sedating types can impair driving; avoid alcohol
  • SJS/TEN survivors: never take the culprit drug again; carry an allergy card or medical alert bracelet; relatives may share genetic risk
  • Methotrexate: take once a week on the same day; avoid alcohol; use reliable contraception
  • Biologics: report fever or infection; avoid live vaccines
7.Complications & Red Flags
ComplicationWhat to watch for
SJS/TENFever, painful skin, blisters, mucosal erosions after a new drug
Airway angioedema / anaphylaxisStridor, hoarseness, wheeze, hypotension
SepsisIn SJS/TEN or erythrodermic psoriasis
Ocular scarringAfter SJS/TEN; uveitis in Behçet
ErythrodermaRed skin over most of the body — heat and fluid loss
Eczema herpeticumPunched-out erosions in atopic dermatitis — urgent antiviral
Scleroderma renal crisisSudden hypertension and kidney failure — treat promptly with an ACE inhibitor; high-dose corticosteroids can trigger it
MalignancyDermatomyositis in adults
8.High-Yield Points
  • Psoriasis: silvery-scaled plaques, Koebner phenomenon; methotrexate is weekly
  • Atopic dermatitis: itch, dry skin — emollients daily
  • Contact dermatitis: ask what the skin touched before symptoms; patch testing
  • Urticaria: second-generation antihistamines first; sedating antihistamines → no driving
  • Angioedema with airway signs → IM epinephrine; ACE inhibitor angioedema → stop the drug
  • SJS under 10%, TEN over 30% BSA; Nikolsky positive; stop the drug, manage like a burn
  • Culprits: allopurinol, carbamazepine (HLA-B*15:02), lamotrigine, phenytoin, sulfonamides
  • SLE: butterfly rash, photosensitivity; lukewarm water, sun protection
  • Raynaud: white → blue → red; keep warm, stop smoking
  • Scleroderma: thick, tight skin, reduced facial expression
  • Dermatomyositis: heliotrope rash, Gottron papules
  • Behçet: oral and genital ulcers, uveitis (dry eye points to Sjögren syndrome instead)

Country Notes

United States

  • Carbamazepine labeling recommends HLA-B*15:02 testing before use in clients with ancestry in genetically at-risk populations, including most Asian populations.

Philippines

  • HLA-B*15:02 is common in parts of the Philippines; carbamazepine and allopurinol are frequent causes of severe drug reactions, so ask about recent use of these drugs in any client with a new blistering rash.
  • Over-the-counter purchase of antibiotics and pain relievers is common; take a detailed medication history including drugs bought without a prescription.

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